Paroxysmal nocturnal hemoglobinuria surgery
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Paroxysmal nocturnal hemoglobinuria Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Surgery
Hematopoietic cell transplantation
- Hematopoietic stem cell transplantation is an important curative therapy for paroxysmal nocturnal hemoglobinuria.
- Stem cell transplantation is usually reserved for the severely affected patients and is indicated for the following type of patients:[1]
- Patients with severe aplastic anemia who are HLA matched donor.
- Patients with myelodysplastic syndromes
- Patients who are unresponsive to the anti complement therapy (eculizumab)
- Transplantation related issues:[2]
- Acute Gravt-Versus-Host Disease (GVHD) occurs in almost third of the PNH patients treated with transplant.
- Survival rate of the PNH patients treated with transplantation is from 50% to 60%.
References
- ↑ DeZern AE, Zahurak M, Symons H, Cooke K, Jones RJ, Brodsky RA (2017). "Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia". Biol Blood Marrow Transplant. 23 (3): 498–504. doi:10.1016/j.bbmt.2016.12.628. PMC 5373094. PMID 28013015.
- ↑ Peffault de Latour R, Schrezenmeier H, Bacigalupo A, Blaise D, de Souza CA, Vigouroux S; et al. (2012). "Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria". Haematologica. 97 (11): 1666–73. doi:10.3324/haematol.2012.062828. PMC 3487438. PMID 22689687.